New stand magnifiers do not meet rated levels of performance

A new range of stand magnifiers has been released by the COIL company in the United Kingdom. Examination of these magnifiers reveals that they fail to deliver the rated magnifications labelled prominently on the appliances, as a result of the manufacturer's conformance with the requirements of the German DIN standard and the use of back vertex power (F'_v) rather than equivalent dioptric power (F_m) of the magnifier. In this study we provide information on the optometric parameters of these new stand magnifiers that will assist the more accurate specification of improvements in vision expected from their use.     

Percutaneous technique for venovenous bypass including a heat exchanger is safe and reliable in liver transplantation

We have introduced and evaluated several modifications of the conventional venovenous bypass (VVBP) in 29 adult patients undergoing liver transplantation (OLT). A percutaneous technique for insertion of a jugular venous return cannula and a femoral vein cannula was applied. The inferior mesenteric vein (IMV) was used for splanchnic decompression, which facilitated dissection of the recipient liver and allowed portal anastomosis to be performed without disconnecting the portal bypass. A heat exchanger was introduced into the bypass circuit to prevent heat loss. The percutaneous technique prevented complications related to dissection in the axilla and groin. Hemodynamic characteristics corresponded to those found using the traditional technique. Complications related to the VVBP were seen in only one patient in whom the femoral catheter was accidentally introduced into the femoral artery. We conclude that percutaneous cannulas, use of the IMV for splanchnic decompression and the introduction of a heat exchanger offer significant benefits and that they are safe and reliable. Received: 23 August 1996 Received after revision: 14 January 1997 Accepted: 27 January 1997     

ORAL-MOTOR DYSFUNCTION AND FEEDING DISORDERS OF INFANTS WITH TURNER SYNDROME

The oral-motor function of 10 infants with Turner syndrome and their age- and sex-matched controls were assessed during feeding. In addition to well-recognised dysmorphic features, including oral anomalies and high-arched palates, index infants had marked hypotonia of the cheeks and lips, dysfunctional tongue movements and poorly developed chewing skills. Their meal-times were significantly shorter than those of the controls and they weighed significantly less at six, 12 and 15 months. All mothers of infants with Turner syndrome complained of difficulties feeding their children and these problems often had been present since birth.     

Laryngotracheal resection and reconstruction for subglottic stenosis.

Eighty patients with inflammatory stenoses of the subglottic larynx and upper trachea were treated by single-stage laryngotracheal resection and reconstruction. Fifty stenoses originated from postintubation lesions (endotracheal tubes, tracheostomy, cricothyroidostomy), 7 originated from trauma, 19 were idiopathic, and 4 were miscellaneous. Repair consisted of resection of the anterolateral cricoid arch in all patients, plus resection of posterior laryngeal stenosis where present, with salvage of the posterior cricoid plate, appropriate resection and tailoring of the trachea, and primary anastomosis using a posterior membranous tracheal wall flap to resurface the bared cricoid cartilage in 31 patients. One postoperative death resulted from acute myocardial infarction. Long-term results were excellent in 18 patients, good in 48, satisfactory in 8, and failure in 2. Three additional patients had good results at discharge but were followed up for less than 6 months.     

Pheochromocytoma and paraganglioma: comparison of MR imaging with CT and I-131 MIBG scintigraphy

To ascertain the magnetic resonance (MR) imaging characteristics of pheochromocytomas and paragangliomas and to compare MR with computed tomography (CT) and iodine-131 metaiodobenzylguanidine (I-131 MIBG), 19 patients (18 with pheochromocytomas, one with a paraganglioma) were studied. The 18 patients with pheochromocytomas had had positive findings with I-131 MIBG scintigraphy. Abdominal pheochromocytomas were generally hypointense compared with normal liver on T1-weighted MR images and extremely hyperintense on T2-weighted MR images. MR imaging was preferable to CT in the evaluation of primary pheochromocytomas due to superior tissue characterization, particularly in the patient with hypertension and borderline catecholamine levels. For patients with recurrent or metastatic disease, the data suggest that I-131 MIBG scintigraphy is the examination of choice.     

Thyroid hormone induction of keratin genes: a two-step activation of gene expression during development

To determine the mechanism of action of the thyroid hormone triiodothyronine (T3) during metamorphosis of the amphibian epidermis, we have investigated the developmental activation of the 63-kD keratin genes in the frog Xenopus laevis. These genes code for three closely related keratins that first appear in the larval epidermis and accumulate during metamorphosis to become the most abundant proteins in the adult epidermis. We report here that the 63-kD keratins and their mRNAs first appear at stages 48-52. The level of 63-kD keratin gene expression remains relatively low until stage 56 and then increases dramatically. Quantitative analysis of the concentration of 63-kD keratin mRNA demonstrates low levels until stage 55/56, followed by an increase greater than 300-fold from stage 55/56 to the adult. Each adult cell contains approximately 55,000 molecules of the 63-kD keratin mRNAs. T3 is not required for the initial activation of the 63-kD keratin genes, but high-level expression is absolutely dependent on T3. High-level expression is prevented by propylthiouracil, which inhibits thyroid hormone synthesis and can be induced precociously both in vivo and in vitro with exogenous T3 as early as stage 48, but not prior to that time. Thus, the full activation of the 63-kD keratin genes during development requires two regulatory steps, one independent and one dependent on T3.     

Chaperonin-mediated assembly of wild-type and mutant subunits of human propionyl-CoA carboxylase expressed in Escherichia coli

We developed a bacterial expression system for the human alpha and beta cDNAs of propionyl-CoA carboxylase (PCC). These cDNAs (less the putative mitochondrial matrix targeting presequences) were co-expressed in Escherichia coli on one plasmid vector with each cDNA having its own IPTG-inducible promoter. Only negligible amounts of active PCC were measured despite the presence of both alpha and beta subunits as indicated by Western blot analysis and the almost complete biotinylation of the alpha subunit. Co-expression of this plasmid with a second plasmid vector over-expressing the E. coli chaperonin proteins, groES and groEL, resulted in a several hundred-fold increase in PCC specific activity, to a level comparable with that found in crude human liver extracts. PCC was partially purified on monomeric avidin affinity resin and the presence of both alpha and beta subunits was demonstrated, thereby confirming the assembly of both subunits into an active enzyme. Deficiency of either alpha PCC or beta PCC results in propionic acidemia, an autosomal recessive disorder. We used this expression system to characterize one missense mutation previously described in five Japanese alleles, namely C1283T (Thr428lle) in beta PCC. This mutation, when expressed in E.coli under the same conditions as that of wild-type PCC, had null activity, despite the presence of assembled alpha PCC and beta PCC subunits. This bacterial expression system can be useful for analysis of either alpha PCC or beta PCC mutations. Our findings indicated that the groES and groEL chaperonin proteins were essential for folding and assembly of the human PCC heteromeric subunits.